Cerebellar and bone metastasis from primary cardiac sarcoma: an unusual case

Abstract

Dear Editor, Primary cardiac tumours are uncommon [3]. The incidence in autopsy series ranged from 0.001 to 0.3 % [5]. About threequarters of these are benign, the other quarter malignant [6]. Because of the few reported cases and poor prognosis of primary cardiac sarcomas, the metastatic potential and behaviour of these tumours are not fully understood [4].We present the case of a 28-year-old male who came acutely to our hospital because of congestive heart failure. He required an emergent thoracotomy to resection of a left atrium mass. The surgical excision was complete. The histological examination showed a diffusely infiltrating mass compatible with a pleomorphic high-grade sarcoma. After heart surgery the patient had chemotherapy and local radiation. One year later he was admitted to the neurosurgical department because of a 10-day history of headache, vomiting and instability. The cranial magnetic resonance imaging revealed a right cerebellar mass with ring contrast-enhancing and surrounding edema that compressed the fourth ventricle. The patient underwent an infratentorial craniectomy with complete resection of the lesion. Histopathologically the tumour was mainly composed of pleomorphic malignant cells with irregular nuclei and prominent nucleoli, areas of necrosis and a significant degree of anaplasia. Tumour cells were inmunoreative for vimentin, desmin, smooth muscle actin and neuronal specific enolase and negative for myo D1, CD99, CD68 and AE1/AE3. It was compatible with a metastasis of pleomorphic undifferentiated sarcoma with a positive muscle marker, suggesting leiomyosarcoma differentiation. The proliferation marker Ki67 was 60 %. The patient had subsequent chemotherapy and radiotherapy. Four years after complaining of left hip pain, the patient underwent hip magnetic resonance imaging, which revealed a well-demarcated mass at the left femoral neck, with heterogeneous gadolinium enhancement. There were no other bone, cardiac or brain lesions in the total body scan. He received palliative radiotherapy for the initial management of pain. Then a left proximal femoral resection and total hip replacement were performed. The histological analysis confirmed an osteolytic lesion composed of atypical giant undifferentiated cells compatible with a metastasis from cardiac pleomorphic sarcoma. More than 6 years after the initial diagnosis, the patient remains alive and healthy. DiscussionPrimary cardiac tumours represent between 5 % and 10 % of all cardiac tumours. The majority are benign, with only a small portion being malignant. Undifferentiated pleomorphic sarcoma is extremely rare. Most have been published as single case reports [6] and minimal epidemiological data have been collected. Usually, they present in young patients and have a poor prognosis, often fatal, considerably worse than that of * Aida Antuña Ramos